Elucidation of preventive effect of amyloidogenesis by low molecule drugs in FAP
Project/Area Number |
25461275
|
Research Category |
Grant-in-Aid for Scientific Research (C)
|
Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Neurology
|
Research Institution | Shinshu University |
Principal Investigator |
YAZAKI Masahide 信州大学, 学術研究院医学系, 准教授 (70372513)
|
Co-Investigator(Renkei-kenkyūsha) |
SEKIJIMA Yoshiki 信州大学, 学術研究院医学系, 准教授 (60322715)
|
Project Period (FY) |
2013-04-01 – 2016-03-31
|
Project Status |
Completed (Fiscal Year 2015)
|
Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2015: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2014: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2013: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000)
|
Keywords | FAP / アミロイドーシス / アミロイド融解 / 低分子薬剤 / トランスサイレチン / 線維融解 / FAP / amyloid turnover / 肝移植 / ジフルニサル / 野生型TTR |
Outline of Final Research Achievements |
We studied the effectiveness of low molecule anti-transthyretin (TTR) amyloidosis drugs in TTR-related familial amyloid polyneuropathy (FAP). In liver-transplanted FAP patients, decrease in amount of amyloid deposits was seen in gastroduodenal amyloid after kiver transplantation. The composition ratios of variant and wild-type TTR fluctuated in serially biopsied samples probably due to suppress amyloidogenesis in both variant and wild-type TTR. In a domino liver-transplant recipient who had been started on oral intake of anti-TTR amyloidosis drug from preclinical stage of transmitted FAP, FAP developed 12 years after operation, suggesting that these drugs might have preventive effect against FAP in domino recipients.
|
Report
(4 results)
Research Products
(77 results)
-
-
-
-
-
-
[Journal Article] The pathological and biochemical identification of possible seed-lesions of transmitted transthyretin amyloidosis after domino liver transplantaion.2015
Author(s)
Yoshinaga, T, Yazaki M, Sekijima Y, Kametani F, Miyashita K, Hachiya N, Tanaka T, Koaudo N, Higuchi K, Ikeda S.
-
Journal Title
J Path: Clin Res 2015
Volume: 1
Issue: 2
Pages: 1-8
DOI
Related Report
Peer Reviewed / Open Access
-
-
[Journal Article] Pathological, biochemical, and biophysical characteristics of the transthyretin variant Y114H (p.Y134H) explain its very mild clinical phenotype2015
Author(s)
Sekijima Y, Campos RI, Hammarstrom P, Nilsson KP, Yoshinaga T, Nagamatsu K, Yazaki M, Kametani F, Ikeda SI.
-
Journal Title
Peripher Nerv Syst
Volume: 20
Issue: 4
Pages: 372-379
DOI
Related Report
Peer Reviewed / Int'l Joint Research / Acknowledgement Compliant
-
-
-
-
[Journal Article] Mechanism for increased hepatic glycerol synthesis in the citrin/mitochondria glycerol-3-phosphate dehydrogenase double-knockout mouse: Urine glycerol and glycerol 3-phosphate as potential diagnostic markers of human citrin deficiency.2015
Author(s)
Moriyama M, Fujimoto Y, Rikimaru S,Ushikai M, Kuroda E, Kawabe K, Takano K,Asakawa A, Inui A, Eto K, Kadowaki T, Sinasac DS, Okano Y, Yazaki M, Ikeda SI,Zhang C, Song YZ,Sakamoto O, Kure S, Mitsubuchi H, Endo F, Horiuchi M, Nakamura Y, Yamamura KI, Saheki T.
-
Journal Title
Biochim Biophys Acta.
Volume: 印刷中
Issue: 9
Pages: 1787-1795
DOI
Related Report
Peer Reviewed / Open Access
-
[Journal Article] Steatogenesis in adult-onset type II citrullinemia is associated with down-regulation of PPARalpha2015
Author(s)
Komatsu M, Kimura T, Yazaki M, Tanaka N, Yang Y, Nakajima T, Horiuchi A, Fang ZZ, Joshita S, Matsumoto A, Umemura T, Tanaka E, Gonzalez FJ, Ikeda S, Aoyama T
-
Journal Title
Biochim Biophys Acta
Volume: 1852
Issue: 3
Pages: 473-481
DOI
Related Report
Peer Reviewed / Int'l Joint Research
-
-
-
-
-
[Journal Article] Identification of disease-promoting HLA class I and protective class II modifiers in the Japanese patients with familial Mediterranean fever.2015
Author(s)
Yasunami M, Nakamura H, Agematsu K, Nakamura A, Yazaki M, Kishida D, Yachie A, Toma T, Masumoto J, Ida H, Koga T, Kawakami A, Eguchi K, Furukawa H, Nakamura T, Nakaumura M, Migita K.
-
Journal Title
NAID
Related Report
Peer Reviewed
-
[Journal Article] Mechanism for increased hepatic glycerol synthesis in the citrin/mitochondrial glycerol-3-phosphate dehydrogenase double-knockout mouse: urine glycerol and glycerol 3-phosphate as potential diagnostic markers of human citrin deficiency2015
Author(s)
Moriyama M, Fujimoto Y, Rikimaru S, Ushikai M, Kuroda E, Kawabe K, Takano K, Asakawa A, Inui A, Eto K, Kadowaki T, Sinasac DS, Okano Y, Yazaki M, Ikeda S, Zhang C, Song YZ, Sakamoto O, Kure S, Mitsubuchi H, Endo F, Horiuchi M, Nakamura Y, Yamamura K, Saheki T.
-
Journal Title
Biochim Biophys Acta.
Volume: 印刷中
Related Report
Peer Reviewed
-
-
[Journal Article] Successful treatment with humanized anti-interleukin-6 receptor antibody (tocilizumab) in a case of AA amyloidosis complicated by familial Mediterranean fever.2015
Author(s)
Hamanoue S, Suwabe T, Hoshino J, Sumida K, Mise K, Hayami N, Sawa N, Takaichi K, Fujii T, Ohashi K, Yazaki M, Ikeda S, Ubara Y.
-
Journal Title
Mod Rheumatol.
Volume: 印刷中
Related Report
Peer Reviewed
-
-
[Journal Article] Familial Mediterranean fever: genotype-phenotype correlations in Japanese patients.2014
Author(s)
Migita K, Agematsu K, Yazaki M, Nonaka F, Nakamura A, Toma T, Kishida D, Uehara R, Nakamura Y, Jiuchi Y, Masumoto J, Furukawa H, Ida H, Terai C, Nakashima Y, Kawakami A, Nakamura T, Eguchi K, Yasunami M, Yachie A.
-
Journal Title
Medicine (Baltimore)
Volume: 93
Issue: 3
Pages: 158-164
DOI
Related Report
Peer Reviewed
-
-
-
-
-
-
-
-
-
[Journal Article] p.E66Q Mutation in the GLA Gene is Associated with a High Risk of Cerebral Small-Vessel Occlusion in Elderly Japanese Males2014
Author(s)
Nakamura K, Sekijima Y, NakamuraK, Hattori K, Nagamatsu K, Shimizu Y, Yazaki M, Sakurai A, Endo F, Fukushima Y, Ikeda S
-
Journal Title
Eur J Neurol
Volume: 21
Issue: 1
Pages: 49-56
DOI
Related Report
Peer Reviewed
-
-
-
[Journal Article] Patient with adult-onset type II citrullinemia beginning 2 years after operation for duodenal malignant somatostatinoma: Indication for liver transplantation2013
Author(s)
Tazawa K, Yazaki M, Fukushima K, Ogata S, Makuuchi M, Morita K, Hiraishi H, Iwasaki Y, Kita J, Kubota K, Ikeda S
-
Journal Title
Hepatol Res
Volume: 43
Issue: 5
Pages: 563-568
DOI
NAID
Related Report
Peer Reviewed
-
[Journal Article] A 73-year-old patient with adult-onset type II citrullinemia successfully treated by sodium pyruvate and arginine.2013
Author(s)
Yazaki M, Kinoshita M, Ogawa S, Fujimi S, Matsushima A, Hineno A, Tazawa K, Fukushima K, Kimura R, Yanagida M, Matsunaga H, Saheki T, Ikeda S.
-
Journal Title
Clin Neurol Neurosurg.
Volume: 115
Issue: 8
Pages: 1542-1545
DOI
Related Report
Peer Reviewed
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-